Delivery of active hexosaminidase across the blood-brain barrier in rats.

Abstract:

:The ability to deliver enzymatically active human hexosaminidase A across the blood-brain barrier and into brain cells of the normal rat was examined. Following osmotic blood-brain barrier modification in the rat, intraarterially administered human hexosaminidase A and B were shown to cross the barrier and enter brain cells. Subcellular fractionation studies demonstrated that most of the human enzyme delivered across the barrier was functionally active and appeared to be inside a subcellular organelle. These studies provide evidence that blood-brain barrier modification permits delivery of functionally active hexosaminidase A into subcellular organelles consistent with that known to be the appropriate site of physiologic activity.

journal_name

Neurology

journal_title

Neurology

authors

Neuwelt EA,Barranger JA,Pagel MA,Quirk JM,Brady RO,Frenkel EP

doi

10.1212/wnl.34.8.1012

subject

Has Abstract

pub_date

1984-08-01 00:00:00

pages

1012-9

issue

8

eissn

0028-3878

issn

1526-632X

journal_volume

34

pub_type

杂志文章
  • Socioeconomic status and disability progression in multiple sclerosis: A multinational study.

    abstract:OBJECTIVE:To examine the association between socioeconomic status (SES) and disability outcomes and progression in multiple sclerosis (MS). METHODS:Health administrative and MS clinical data were linked for 2 cohorts of patients with MS in British Columbia (Canada) and South East Wales (UK). SES was measured at MS sym...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000007190

    authors: Harding KE,Wardle M,Carruthers R,Robertson N,Zhu F,Kingwell E,Tremlett H

    更新日期:2019-03-26 00:00:00

  • Neuroinflammation drives anxiety and depression in relapsing-remitting multiple sclerosis.

    abstract:OBJECTIVE:To explore the inflammatory processes in the pathogenesis of psychiatric symptoms and the prognostic value of psychiatric comorbidities in multiple sclerosis (MS). METHODS:Four hundred five patients with relapsing-remitting (RR) MS underwent psychiatric evaluation by means of Beck Depression Inventory II (BD...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000004411

    authors: Rossi S,Studer V,Motta C,Polidoro S,Perugini J,Macchiarulo G,Giovannetti AM,Pareja-Gutierrez L,Calò A,Colonna I,Furlan R,Martino G,Centonze D

    更新日期:2017-09-26 00:00:00

  • Comparison of Phenotypic Characteristics and Prognosis Between Black and White Patients in a Tertiary ALS Clinic.

    abstract:OBJECTIVE:To compare characteristics between Black and White patients with amyotrophic lateral sclerosis (ALS) in order to identify disparities and phenotypic variability. METHODS:We performed database review for patients seen between 1997 and 2020 at the Emory ALS Center in Atlanta, Georgia. Patients with ALS were in...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000011396

    authors: Brand D,Polak M,Glass JD,Fournier CN

    更新日期:2020-12-28 00:00:00

  • Spectrum of SCN1A mutations in severe myoclonic epilepsy of infancy.

    abstract:OBJECTIVES:SCN1A mutations were recently reported in several patients with severe myoclonic epilepsy in infancy (SMEI). The authors analyzed SCN1A mutations in 93 patients with SMEI and made genotype-phenotype correlation to clarify the role of this gene in the etiology of SMEI. METHODS:All patients fulfilled the crit...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000069463.41870.2f

    authors: Nabbout R,Gennaro E,Dalla Bernardina B,Dulac O,Madia F,Bertini E,Capovilla G,Chiron C,Cristofori G,Elia M,Fontana E,Gaggero R,Granata T,Guerrini R,Loi M,La Selva L,Lispi ML,Matricardi A,Romeo A,Tzolas V,Valseriati

    更新日期:2003-06-24 00:00:00

  • Role of common and rare APP DNA sequence variants in Alzheimer disease.

    abstract:OBJECTIVES:More than 30 different rare mutations, including copy number variants (CNVs), in the amyloid precursor protein gene (APP) cause early-onset familial Alzheimer disease (EOFAD), whereas the contribution of common APP variants to disease risk remains controversial. In this study we systematically assessed the r...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e3182515972

    authors: Hooli BV,Mohapatra G,Mattheisen M,Parrado AR,Roehr JT,Shen Y,Gusella JF,Moir R,Saunders AJ,Lange C,Tanzi RE,Bertram L

    更新日期:2012-04-17 00:00:00

  • Brain white matter volume abnormalities in Lesch-Nyhan disease and its variants.

    abstract:OBJECTIVE:We sought to examine brain white matter abnormalities based on MRI in adults with Lesch-Nyhan disease (LND) or an attenuated variant (LNV) of this rare, X-linked neurodevelopmental disorder of purine metabolism. METHODS:In this observational study, we compared 21 adults with LND, 17 with LNV, and 33 age-, se...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000001128

    authors: Schretlen DJ,Varvaris M,Vannorsdall TD,Gordon B,Harris JC,Jinnah HA

    更新日期:2015-01-13 00:00:00

  • Neurovascular decoupling is associated with severity of cerebral amyloid angiopathy.

    abstract:OBJECTIVES:We used functional MRI (fMRI), transcranial Doppler ultrasound, and visual evoked potentials (VEPs) to determine the nature of blood flow responses to functional brain activity and carbon dioxide (CO2) inhalation in patients with cerebral amyloid angiopathy (CAA), and their association with markers of CAA se...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000435291.49598.54

    authors: Peca S,McCreary CR,Donaldson E,Kumarpillai G,Shobha N,Sanchez K,Charlton A,Steinback CD,Beaudin AE,Flück D,Pillay N,Fick GH,Poulin MJ,Frayne R,Goodyear BG,Smith EE

    更新日期:2013-11-05 00:00:00

  • Leukoencephalopathy induced by levamisole alone for the treatment of recurrent aphthous ulcers.

    abstract::We report imaging and clinical findings of leukoencephalopathy occurring after levamisole. The lesions were hypoattenuating on CT and appeared as multifocal oval or elliptical foci in the white matter or along the subependymal veins on MRI. Most lesions resolve after prompt withdrawal of levamisole. Detailed history t...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000237344.06122.79

    authors: Liu HM,Hsieh WJ,Yang CC,Wu VC,Wu KD

    更新日期:2006-09-26 00:00:00

  • Retrievable stents, "stentrievers," for endovascular acute ischemic stroke therapy.

    abstract::Endovascular therapy for acute ischemic stroke continues to evolve to improve both efficacy and safety. In the late 1990 s, intra-arterial chemical thrombolysis with prourokinase was shown to be effective in achieving partial recanalization and improving clinical outcome, in comparison with intra-arterial heparin admi...

    journal_title:Neurology

    pub_type: 杂志文章,评审

    doi:10.1212/WNL.0b013e3182697e9e

    authors: Novakovic RL,Toth G,Narayanan S,Zaidat OO

    更新日期:2012-09-25 00:00:00

  • Short-term outcomes of skull fracture: a population-based study of survival and neurologic complications.

    abstract::All Olmsted County, Minnesota, residents who experienced brain injury from 1935 through 1979 were identified and their medical records reviewed for survival and neurologic outcome. Minimum inclusion criteria included loss of consciousness or post-traumatic amnesia or neurologic evidence of brain injury or skull fractu...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.39.1.96

    authors: Wiederholt WC,Melton LJ 3rd,Annegers JF,Grabow JD,Laws ER Jr,Ilstrup DM

    更新日期:1989-01-01 00:00:00

  • Comorbidity is associated with disease activity in MS: Findings from the CombiRx trial.

    abstract:OBJECTIVE:To determine whether comorbidity is associated with clinical (relapses, disability worsening) and MRI outcomes in multiple sclerosis (MS) by conducting a secondary analysis of the CombiRx clinical trial. METHODS:CombiRx compared interferon beta-1a, glatiramer acetate, and the combination of these agents. For...

    journal_title:Neurology

    pub_type: 杂志文章,随机对照试验

    doi:10.1212/WNL.0000000000010024

    authors: Salter A,Kowalec K,Fitzgerald KC,Cutter G,Marrie RA

    更新日期:2020-08-04 00:00:00

  • Caregiving in progressive supranuclear palsy.

    abstract:BACKGROUND:Basic issues regarding factors influencing progressive supranuclear palsy (PSP) patient caregiver burden remain unresolved, including whether and how disease severity and duration influence caregiver burden. OBJECTIVE:To examine the relation between PSP patient caregiver burden and disease severity, describ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.51.5.1303

    authors: Uttl B,Santacruz P,Litvan I,Grafman J

    更新日期:1998-11-01 00:00:00

  • Hospital volume and stroke outcome: does it matter?

    abstract:BACKGROUND:Although hospital-outcome relationships have been explored for a variety of procedures and interventions, little is known about the association between annual stroke admission volumes and stroke mortality. Our aim was to determine whether facility type and hospital volume was associated with stroke mortality...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000268485.93349.58

    authors: Saposnik G,Baibergenova A,O'Donnell M,Hill MD,Kapral MK,Hachinski V,Stroke Outcome Research Canada (SORCan) Working Group.

    更新日期:2007-09-11 00:00:00

  • Coexistence of Lambert-Eaton myasthenic syndrome and subacute cerebellar degeneration: differential effects of treatment.

    abstract::A 61-year-old woman presented with two paraneoplastic neurologic disorders--Lambert-Eaton myasthenic syndrome (LEMS) and subacute cerebellar degeneration (SCD)--that antedated the diagnosis of small-cell carcinoma of the lung by 15 months. Plasmapheresis initiated before the identification of the tumor had a beneficia...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.41.10.1682

    authors: Blumenfeld AM,Recht LD,Chad DA,DeGirolami U,Griffin T,Jaeckle KA

    更新日期:1991-10-01 00:00:00

  • Acoustic tumors: atypical features and recent diagnostic tests.

    abstract::About one-third of patients with acoustic tumor (AT) initially seek medical attention for nonaudiologic complaints. The nonspecific early symptoms of AT require the inclusion of AT in many neurologic differential diagnoses. Advances in electrophysiologic and radiographic tests have allowed earlier diagnosis of AT at a...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.33.2.211

    authors: Hart RG,Gardner DP,Howieson J

    更新日期:1983-02-01 00:00:00

  • Functional paralysis of horizontal gaze.

    abstract::A 52-year-old steelworker displayed a consistent defect in leftward voluntary gaze across the midline. Both fast (saccadic) and slow (pursuit) eye movements were affected. Neuro-ophthalmologic evaluation revealed no additional neurologic or ophthalmologic deficit. Pupilary miosis occurred during leftward gaze attempt,...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.29.1.82

    authors: Troost BT,Troost EG

    更新日期:1979-01-01 00:00:00

  • Cerebral microbleeds are a risk factor for warfarin-related intracerebral hemorrhage.

    abstract:BACKGROUND:Cerebral microbleeds are known to be indicative of bleeding-prone microangiopathy and may predict incident intracerebral hemorrhage (ICH). In this study, we investigated whether microbleeds are associated with the incidence of warfarin-related ICH. METHODS:Twenty-four patients with ICH while on outpatient t...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000339060.11702.dd

    authors: Lee SH,Ryu WS,Roh JK

    更新日期:2009-01-13 00:00:00

  • Familial aggregation of amyotrophic lateral sclerosis, dementia, and Parkinson's disease: evidence of shared genetic susceptibility.

    abstract::Clinicians have long suspected an association of classic amyotrophic lateral sclerosis (ALS) with Parkinson's disease (PD), dementia, or both. If proven, this would raise the possibility of a shared genetic susceptibility to the three disorders. To investigate this hypothesis, we compared 151 newly diagnosed ALS patie...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.44.10.1872

    authors: Majoor-Krakauer D,Ottman R,Johnson WG,Rowland LP

    更新日期:1994-10-01 00:00:00

  • Firing rates of human motor units in partially denervated muscle.

    abstract::Single motor unit firing rates were measured from the first dorsal interosseous muscle (FDI) of normal subjects and patients with partial denervation of that muscle. Motor unit discharges were recorded at various levels of voluntary, stationary, isometric contraction of the FDI. The mean increase in firing rate associ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.28.12.1241

    authors: Miller RG,Sherratt M

    更新日期:1978-12-01 00:00:00

  • Cerebral hemodynamics in the diagnosis of normal pressure hydrocephalus.

    abstract::We studied the effect of CSF drainage on cerebral blood flow (CBF) in normal pressure hydrocephalus (NPH) and non-NPH dementia using the 133Xenon inhalation technique. Dementia patients had lower CBF than matched elderly normals. Flow values for NPH and non-NPH patients did not differ before or after CSF drainage. CBF...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.34.1.96

    authors: Kushner M,Younkin D,Weinberger J,Hurtig H,Goldberg H,Reivich M

    更新日期:1984-01-01 00:00:00

  • Evaluation of hyperacute infarct volume using ASPECTS and brain CT perfusion core volume.

    abstract:OBJECTIVE:To compare the accuracy of Alberta Stroke Program Early Computed Tomography Score (ASPECTS) and CT perfusion to detect established infarction in acute anterior circulation stroke. METHODS:We performed an observational study in 59 acute anterior circulation ischemic stroke patients who underwent brain noncont...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000004028

    authors: Demeestere J,Garcia-Esperon C,Garcia-Bermejo P,Ombelet F,McElduff P,Bivard A,Parsons M,Levi C

    更新日期:2017-06-13 00:00:00

  • Absence of IgG1 response in the cerebrospinal fluid of relapsing neuromyelitis optica.

    abstract::The authors studied immunoglobulin (Ig) G subclasses in the CSF and sera of patients with relapsing neuromyelitis optica (RNMO) and typical multiple sclerosis (MS). Although the total IgG concentrations were elevated in the CSF of patients with RNMO and MS, IgG1% and IgG1 index were significantly elevated only in pati...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.62.1.144

    authors: Nakashima I,Fujihara K,Fujimori J,Narikawa K,Misu T,Itoyama Y

    更新日期:2004-01-13 00:00:00

  • Avoidance of dyskinesia: preclinical evidence for continuous dopaminergic stimulation.

    abstract::Current concepts suggest that avoidance of pulsatile stimulation of dopamine receptors in Parkinson's disease (PD) can prevent the onset of dyskinesia. In MPTP-treated primates, repeated administration of levodopa or other short-acting dopamine agonist drugs leads to the onset of marked involuntary movements. In contr...

    journal_title:Neurology

    pub_type: 杂志文章,评审

    doi:10.1212/wnl.62.1_suppl_1.s47

    authors: Jenner P

    更新日期:2004-01-13 00:00:00

  • Impact of APOE genotype on neuropathologic and neurochemical markers of Alzheimer disease.

    abstract:BACKGROUND:The APOE epsilon4 allele has emerged as a major genetic factor for Alzheimer disease (AD), and its presence has been associated with an increase in beta-amyloid senile plaques (SPs) and neuritic plaques (NPs). Whether it affects neurofibrillary tangle (NFT) accumulation or cholinergic losses in AD remains co...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000128091.92139.0f

    authors: Tiraboschi P,Hansen LA,Masliah E,Alford M,Thal LJ,Corey-Bloom J

    更新日期:2004-06-08 00:00:00

  • Autoantibodies in postinfectious acute cerebellar ataxia.

    abstract::The authors found serum immunoglobulin M (IgM) autoantibody in a patient with typical acute cerebellar ataxia (ACA) and identified the antigen molecule as triosephosphate isomerase (TPI). TPI antigenicity to the patient's antibody was the highest in the cerebellar tissue. Eight of 23 patients with ACA had increased Ig...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000178802.38268.1e

    authors: Uchibori A,Sakuta M,Kusunoki S,Chiba A

    更新日期:2005-10-11 00:00:00

  • Alteration of calcium transport in Duchenne erythrocytes.

    abstract::Erythrocyte calcium transport as a function of free [Ca++] was examined using inside-out vesicles from eight pairs of patients with Duchenne dystrophy and age- and sex-matched controls. The Km for Ca++ of the transport protein and the Vmax were higher in the Duchenne vesicles in seven of the eight pairs. The mean Km a...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.30.11.1236

    authors: Mollman JE,Cardenas JC,Pleasure DE

    更新日期:1980-11-01 00:00:00

  • Hemiballism and metastatic brain tumor.

    abstract::We describe a 78-year-old man with right hemiballism as the presenting symptom of a metastatic occult adenocarcinoma of the lung. CT demonstrated two contrast-enhancing lesions: one in the left subthalamic region and the other in the left parietal region. Metastatic tumor was confirmed at autopsy. ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.34.2.204

    authors: Glass JP,Jankovic J,Borit A

    更新日期:1984-02-01 00:00:00

  • Optic nerve edema as a consequence of respiratory disease.

    abstract::The authors describe a patient with bilateral papilledema, visual field abnormalities, poorly reactive pupils, meningeal enhancement on cranial MRI, and diffuse brain parenchymal hypervascularity. The opening pressure at the time of lumbar puncture was normal, and results of other CSF studies were normal. All abnormal...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.53.9.2204

    authors: O'Halloran HS,Berger JR,Baker RS,Lee WB,Pearson PA,Ryan S

    更新日期:1999-12-10 00:00:00

  • Imipramine-mediated interference with levodopa absorption from the gastrointestinal tract in man.

    abstract::The effect of imipramine on the absorption of a single dose of levodopa was studied in male volunteers. By delaying gastric emptying and retarding delivery to intestinal absorptive sites, imipramine interfered with the absorption of levodopa. This action caused by the anticholinergic effect of imipramine. The retardat...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.25.11.1029

    authors: Morgan JP,Rivera-Calimlim L,Messiha F,Sundaresan PR,Trabert N

    更新日期:1975-11-01 00:00:00

  • Acute disseminated encephalomyelitis: a follow-up study of 40 adult patients.

    abstract:OBJECTIVES:To describe the clinical, CSF, and radiologic findings and long-term follow-up in a cohort of patients with acute disseminated encephalomyelitis (ADEM), and to determine possible prognostic factors for progression to MS. METHODS:Forty adults (28 women, mean age 33.5 years) diagnosed with ADEM were analyzed....

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.56.10.1313

    authors: Schwarz S,Mohr A,Knauth M,Wildemann B,Storch-Hagenlocher B

    更新日期:2001-05-22 00:00:00